Clinical spectrum of the pseudotumor cerebri complex in children

Daniel Tibussek1, Dominik T Schneider, Nicola Vandemeulebroecke

  • 1Department of General Pediatrics, University Children's Hospital, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Germany.

Insights

Pseudotumor cerebri (PTC) in children is underrecognized. Improved diagnosis and management strategies are crucial, especially considering potential relapses and associated conditions.

Area of Science:

  • Pediatric Neurology
  • Ophthalmology
  • Neurosurgery

Background:

  • Pseudotumor cerebri (PTC), or idiopathic intracranial hypertension, is a significant neurological disorder in children.
  • Accurate diagnosis and effective management are essential for preventing long-term complications.

Purpose of the Study:

  • To enhance the diagnosis and management protocols for pediatric pseudotumor cerebri (PTC).
  • To identify key considerations for improving patient outcomes in children with PTC.

Main Methods:

  • A comprehensive analysis of 53 pediatric patients with PTC.
  • Evaluation included epidemiology, diagnostic work-up, therapeutic interventions, and clinical follow-up.

Main Results:

  • PTC presentation in children can be subtle, with absent or incomplete papilledema.
  • Cerebrospinal fluid (CSF) opening pressure measurements may be unreliable due to sedation.
  • Normal pediatric CSF pressure ranges are not well-defined; diagnosis may occur below 20 cm H2O.
  • Associated conditions are common, though causality is often unclear.
  • Disease relapse occurs in 20% of cases, highlighting the need for standardized follow-up.

Conclusions:

  • PTC is an important, potentially underdiagnosed condition in pediatric populations.
  • Thorough diagnostic evaluation is vital to uncover treatable underlying conditions.
  • Standardized follow-up programs are essential due to the significant risk of relapse.
Abstract

Related Concept Videos

Increased Intracranial Pressure l: Introduction01:14

Increased Intracranial Pressure l: Introduction

Intracranial hypertension is a sustained elevation of intracranial pressure (ICP) above 22 mm Hg. In supine adults, normal ICP is ~7–15 mm Hg.The rigid, nonexpandable cranium contains three components—brain tissue, blood, and cerebrospinal fluid (CSF)—that total ~1,700 mL in a typical adult: 1,400 mL brain (~80%), 150 mL blood (~10%), and 150 mL CSF (~10%). According to the Monro–Kellie doctrine, total intracranial volume is effectively fixed. When one component expands, CSF and venous blood...
Increased Intracranial Pressure ll: Pathophysiology01:29

Increased Intracranial Pressure ll: Pathophysiology

Increased intracranial pressure (ICP) refers to a potentially life-threatening rise in pressure inside the skull. This usually happens when there is a major change in the volume of brain tissue, blood, or cerebrospinal fluid (CSF) — the three components inside the skull. According to the Monro-Kellie doctrine, if the volume of one component increases, the volumes of the other components must decrease to maintain normal pressure. If this does not happen, ICP rises.The process often begins with...
Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial infections,...
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Seizures: Classification01:13

Seizures: Classification

Epilepsy is primarily characterized by unpredictable seizures, either provoked by an identifiable factor, such as injury or illness, or unprovoked, occurring spontaneously without apparent cause.
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types: